Multifocal motor neuropathy
- Dale Lange, MD
Dale Lange, MD
- Professor of Neurology
- Weill College of Medicine at Cornell University
- Jessica Robinson-Papp, MD
Jessica Robinson-Papp, MD
- Assistant Professor
- Icahn School of Medicine at Mount Sinai
Multifocal motor neuropathy (MMN), also known as multifocal motor neuropathy with conduction block, is a rare neuropathy characterized by progressive asymmetric weakness and atrophy without sensory abnormalities, a presentation similar to that of motor neuron disease. This review will discuss the clinical aspects of MMN. Other immune-mediated neuropathies are discussed separately. (See "Immune-mediated neuropathies".)
MMN is considered an immune-mediated disorder given the following observations [1-3]:
●Clinical improvement with intravenous immune globulin therapy
●The presence of anti-GM1 antibodies in many but not all patients
●A perivascular lymphocytic infiltration observed on nerve biopsy in some reports
- So YT. Immune-mediated neuropathies. Continuum (Minneap Minn) 2012; 18:85.
- Lange DJ, Trojaborg W. Do GM1 antibodies induce demyelination? Muscle Nerve 1994; 17:105.
- Muley SA, Parry GJ. Multifocal motor neuropathy. J Clin Neurosci 2012; 19:1201.
- Nowacek DG, Teener JW. Multifocal motor neuropathy. Semin Neurol 2012; 32:500.
- Taylor BV, Dyck PJ, Engelstad J, et al. Multifocal motor neuropathy: pathologic alterations at the site of conduction block. J Neuropathol Exp Neurol 2004; 63:129.
- Uncini A, Kuwabara S. Nodopathies of the peripheral nerve: an emerging concept. J Neurol Neurosurg Psychiatry 2015; 86:1186.
- Cats EA, van der Pol WL, Piepers S, et al. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology 2010; 75:818.
- Pestronk A, Cornblath DR, Ilyas AA, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 1988; 24:73.
- Slee M, Selvan A, Donaghy M. Multifocal motor neuropathy: the diagnostic spectrum and response to treatment. Neurology 2007; 69:1680.
- Meuth SG, Kleinschnitz C. Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options. Eur Neurol 2010; 63:193.
- Kornberg AJ, Pestronk A. The clinical and diagnostic role of anti-GM1 antibody testing. Muscle Nerve 1994; 17:100.
- Pestronk A. Invited review: motor neuropathies, motor neuron disorders, and antiglycolipid antibodies. Muscle Nerve 1991; 14:927.
- Pakiam AS, Parry GJ. Multifocal motor neuropathy without overt conduction block. Muscle Nerve 1998; 21:243.
- Katz JS, Barohn RJ, Kojan S, et al. Axonal multifocal motor neuropathy without conduction block or other features of demyelination. Neurology 2002; 58:615.
- Delmont E, Azulay JP, Giorgi R, et al. Multifocal motor neuropathy with and without conduction block: a single entity? Neurology 2006; 67:592.
- Nobile-Orazio E, Cappellari A, Meucci N, et al. Multifocal motor neuropathy: clinical and immunological features and response to IVIg in relation to the presence and degree of motor conduction block. J Neurol Neurosurg Psychiatry 2002; 72:761.
- Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J Peripher Nerv Syst 2010; 15:295.
- Léger JM, Chassande B, Musset L, et al. Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-controlled study. Brain 2001; 124:145.
- Van den Berg LH, Kerkhoff H, Oey PL, et al. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry 1995; 59:248.
- Federico P, Zochodne DW, Hahn AF, et al. Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study. Neurology 2000; 55:1256.
- Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2012; 78:1009.
- Nobile-Orazio E, Gallia F. Multifocal motor neuropathy: current therapies and novel strategies. Drugs 2013; 73:397.
- Feldman EL, Bromberg MB, Albers JW, Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 1991; 30:397.
- Carpo M, Cappellari A, Mora G, et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology 1998; 50:1480.
- van Schaik IN, van den Berg LH, de Haan R, Vermeulen M. Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev 2005; :CD004429.
- Azulay JP, Blin O, Pouget J, et al. Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study. Neurology 1994; 44:429.
- Racosta JM, Sposato LA, Kimpinski K. Subcutaneous versus intravenous immunoglobulin for chronic autoimmune neuropathies: A meta-analysis. Muscle Nerve 2017; 55:802.
- Umapathi T, Hughes RA, Nobile-Orazio E, Léger JM. Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy. Cochrane Database Syst Rev 2015; :CD003217.
- Brannagan TH 3rd, Alaedini A, Gladstone DE. High-dose cyclophosphamide without stem cell rescue for refractory multifocal motor neuropathy. Muscle Nerve 2006; 34:246.
- Rüegg SJ, Fuhr P, Steck AJ. Rituximab stabilizes multifocal motor neuropathy increasingly less responsive to IVIg. Neurology 2004; 63:2178.
- Stieglbauer K, Topakian R, Hinterberger G, Aichner FT. Beneficial effect of rituximab monotherapy in multifocal motor neuropathy. Neuromuscul Disord 2009; 19:473.
- Chaudhry V, Cornblath DR. An open-label trial of rituximab (Rituxan®) in multifocal motor neuropathy. J Peripher Nerv Syst 2010; 15:196.
- Lange DJ, Weimer LH, Trojaborg W, et al. Multifocal motor neuropathy with conduction block: slow but not benign. Arch Neurol 2006; 63:1778.